A follow-up of 254 patients with intrathoracic sarcoidosis has been carried out after a median of 27 years from diagnosis. All patients could be traced. No excess mortality was found (80 deaths observed versus 66 expected). The causes of death were related to sarcoidosis in 41%. X-ray stage was related to survival with a significant excess mortality for stage 3. Early clearing of X-ray changes gave a favourable vital prognosis. Survival was related to lung function, especially forced expiratory volume in 1 second (FEV1) and total lung capacity (TLC). All deaths from sarcoidosis were seen among patients with respiratory symptoms at presentation. For sarcoidosis & sarcoidosis-related deaths (N = 33), presence of one or more of the respiratory symptoms increased the risk significantly. For overall mortality respiratory symptoms were also significant predictors; this was the case even after adjusting for age, sex, x-ray stage and lung function (FEV1 & TLC) at presentation. In conclusion, no excess mortality was observed after a median of 27 years follow-up. Lung function, respiratory symptoms and to some extent X-ray stage at time of diagnosis influenced long-term mortality in patients with intrathoracic sarcoidosis.